Quaglino D, Di Leonardo G, Stati M
Clinica Medica II, Università degli Studi de L'Aquila, Italia.
Acta Haematol. 2001;106(3):122-5. doi: 10.1159/000046601.
The authors describe a case of thrombocythaemia, with subsequent leukaemic transformation. Cytochemical and immunocytochemical investigations indicated a trilineage involvement of the myeloid series, compatible with a leukaemic transformation at the level of the colony-forming unit granulocytes, erythrocytes, macrophages, megakaryocytes. No cytogenetic abnormalities were observed. The criteria which have been proposed to differentiate essential thrombocythaemia from pre-fibrotic thrombocythaemia, as an early phase of idiopathic myelofibrosis, are discussed. The differentiation is not only of academic interest but has relevant practical implications, since survival in the two conditions is significantly different. The possible significance of an accompanying monoclonal gammopathy is discussed.
作者描述了一例血小板增多症，随后发生白血病转化。细胞化学和免疫细胞化学研究表明髓系呈三系受累，这与粒细胞、红细胞、巨噬细胞、巨核细胞集落形成单位水平的白血病转化相符。未观察到细胞遗传学异常。讨论了为区分原发性血小板增多症与作为原发性骨髓纤维化早期阶段的纤维化前血小板增多症而提出的标准。这种区分不仅具有学术意义，而且具有实际的重要意义，因为这两种情况的生存率有显著差异。还讨论了伴发单克隆丙种球蛋白病的可能意义。
